Marklund S L, Heiskala H, Westermarck T, Santavuori P
Clin Sci (Lond). 1986 Jul;71(1):57-60. doi: 10.1042/cs0710057.
The neuronal ceroid-lipofuscinoses is a group of diseases characterized by a widespread accumulation in the body of pigments believed to be end-products of lipid-peroxidation damaged organelles. It was recently shown that cerebrospinal fluid from patients with infantile and juvenile neuronal ceroid-lipofuscinosis were less protective against superoxide radical-induced hydroxyl radical formation compared with controls. The content of superoxide dismutase isoenzymes in cerebrospinal fluid and in plasma from patients with different forms of neuronal ceroid-lipofuscinosis was analysed. No significant difference from controls could be demonstrated in samples from patients with juvenile neuronal ceroid-lipofuscinosis. The few samples from patients with infantile and late infantile neuronal ceroid-lipofuscinosis analysed all fell within the range defined by the controls.
神经元蜡样脂褐质沉积症是一组疾病，其特征是身体中广泛积累被认为是脂质过氧化损伤细胞器终产物的色素。最近研究表明，与对照组相比，患有婴儿型和青少年型神经元蜡样脂褐质沉积症患者的脑脊液对超氧自由基诱导的羟自由基形成的保护作用较弱。分析了不同形式神经元蜡样脂褐质沉积症患者脑脊液和血浆中超氧化物歧化酶同工酶的含量。青少年型神经元蜡样脂褐质沉积症患者的样本与对照组相比无显著差异。所分析的少数婴儿型和晚婴儿型神经元蜡样脂褐质沉积症患者的样本均在对照组定义的范围内。
